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Medulloblastoma is a most common pediatric malignant primary brain tumor (cancer) originating in the part of the brain that is towards the back and the bottom, on the floor of the skull, in the cerebellum or posterior fossa.

The brain is divided into two main parts, the larger cerebrum on top and the smaller cerebellum below towards the back. They are separated by a membrane called the tentorium. Tumors that originate in the cerebellum or the surrounding region below the tentorium are therefore called infratentorial.

Historically medulloblastoma's have been classified as a PNET (primitive neuroectodermal tumour), however it is now known that medulloblastoma is distinct from supratentorial PNET's and are no longer considered similar entities.^[1]

Medulloblastoma's are non-invasive rapidly growing tumors that, unlike most brain tumors, spread through the cerebrospinal fluid (CSF) and frequently metastasize to different locations along the surface of the brain and spinal cord.

The cumulative relative survival rate for all age groups and histology follow-up was 60%, 52%, and 32% at 5 years, 10 years, and 20 years, respectively, with children doing better than adults.^[2]

Signs and symptoms

Signs and symptoms are mainly due to secondary increased intracranial pressure due to blockage of the fourth ventricle and are usually present for 1 to 5 months before diagnosis is made. The child typically becomes listless, with repeated episodes of vomiting, and a morning headache, which may lead to a misdiagnosis of gastrointestinal disease or migraine. Soon after, the child will develop a stumbling gait, frequent falls, diplopia, papilledema, and sixth cranial nerve palsy. Positional dizziness and nystagmus are also frequent and facial sensory loss or motor weakness may be present. Decerebrate attacks appear late in the disease.

Extraneural metastasis to the rest of the body is rare, and when it occurs is in the setting of relapse, more commonly in the era prior to routine chemotherapy.

Pathogenesis

Medulloblastomas usually form in the vicinity of the fourth ventricle, between the brainstem and the cerebellum. Tumors with similar appearance and characteristics originate in other parts of the brain, but they are not identical to medulloblastoma.^[3]

Although it is thought that medulloblastomas originate from immature or embryonal cells at their earliest stage of development, the cell of origin depends on the subgroup of medulloblastoma. WNT tumours originate from the lower rhombic lip of the brainstem while SHH tumours originate from the external granular layer.^{[citation needed]}

It is currently thought that medulloblastoma arises from cerebellar stem cells that have been prevented from dividing and differentiating into their normal cell types. This accounts for the varying histologic variants seen on biopsy. Both perivascular pseudorosette and Homer-Wright rosette pseudorosettes formation are highly characteristic of medulloblastoma and is seen in up to half of the cases. Homer-Wright rosettes are pseudorosettes consisting of tumor cells surrounding a fibrillar area.^[4] Also, the classic rosette with tumor cells around a central lumen can be seen.^[5]

Recent integrated genomic studies have revealed that medulloblastoma is composed of four distinct molecular and clinical variants termed WNT, SHH, Group 3 and Group 4.^[6] Of these subgroups WNT patients have an excellent prognosis and Group 3 have a dismal prognosis. There also exists subgroup specific alternative splicing which further confirms the existence of distinct subgroups and highlights the transcriptional heterogeneity between subgroups.^[7] Medulloblastomas are also seen in Gorlin syndrome as well as Turcot syndrome. Recurrent mutations in the genes CTNNB1, PTCH1, MLL2, SMARCA4, DDX3X, CTDNEP1, KDM6A and TBR1 were identified in individuals with medulloblastoma.^[8]

Diagnosis

The tumor is distinctive on T1 and T2-weighted MRI with heterogeneous enhancement and typical location adjacent to and extension into the fourth ventricle.

Histologically, the tumor is solid, pink-gray in color, and is well circumscribed. The tumor is very cellular, many mitoses, little cytoplasm, and has the tendency to form clusters and rosettes.

Correct diagnosis of medulloblastoma may require ruling out atypical teratoid rhabdoid tumor (ATRT)^[9]

Cerebellar medulloblastoma in an adult
Cerebellar medulloblastoma in an adult

Treatment

Treatment begins with maximal surgical removal of the tumor. The addition of radiation to the entire neuraxis and chemotherapy may increase the disease-free survival. There is some evidence that proton beam irradiation reduces the impact of radiation on the cochlear and cardiovascular areas and reduces the cognitive late effects of cranial irradiation.^[10]^[11] This combination may permit a 5 year survival in more than 80% of cases. The presence of desmoplastic features such as connective tissue formation offers a better prognosis. Prognosis is worse if the child is less than 3 years old, there is an inadequate degree of resection, or if there is any CSF, spinal, supratentorial or systemic spread. Dementia after radiotherapy and chemotherapy is a common outcome appearing two to four years following treatment.

Increased intracranial pressure may be controlled with corticosteroids or a ventriculoperitoneal shunt.

Chemotherapy

Chemotherapy is often used as part of treatment. Evidence of benefit however is not clear as of 2013.^[12] There are a couple of different chemotheraputic regimens for medulloblastoma, but most involve a combination of lomustine, cisplatin, carboplatin, vincristine or cyclophosphamide. In younger patients (less than 3–4 years of age), chemotherapy can delay, or in some cases possibly even eliminate, the need for radiotherapy. However, both chemotherapy and radiotherapy often have long-term toxicity effects, including delays in physical and cognitive development, higher risk of second cancers and increased cardiac disease risks.^[13]^[14]

Outcomes

Array-based karyotyping of 260 medulloblastomas by Pfister S, et al. resulted in the following clinical subgroups based on cytogenetic profiles:^[15]

Poor prognosis: gain of 6q or amplification of MYC or MYCN
Intermediate: gain of 17q or an i(17q) without gain of 6q or amplfication of MYC or MYCN
Excellent prognosis: 6q and 17q balanced or 6q deletion

Transcriptional profiling shows the existence of four main subgroups (Wnt, Shh, Group 3, and Group 4).^[16]

Very good prognosis: WNT group, CTNNB1 mutation
Infants good prognosis, others intermediate: SHH group, PTCH1/SMO/SUFU mutation, GLI2 amplification, or MYCN amplification
Poor prognosis: Group 3, MYC amplification, photoreceptor/GABAergic gene expression
Intermediate prognosis: Group 4, gene expression of neuronal/glutamatergic, CDK6 amplification, MYCN amplification

Survival

The cumulative relative survival rate for all age groups and histology follow-up was 60%, 52%, and 47% at 5 years, 10 years, and 20 years, respectively. Patients diagnosed with a medulloblastoma or PNET are 50 times more likely to die than a matched member of the general population. The most recent population-based (SEER) 5-year relative survival rates are 69% overall, but 72% in children (1–9 years) and 67% in adults (20+ years). The 20 year survival rate is 51% in children. Children and adults have different survival profiles, with adults faring worse than children only after the 4th year post-diagnosis (after controlling for increased background mortality). Before the 4th year, survival probabilities are nearly identical.^[2] Longterm sequelae of standard treatment include hypothalamic-pituitary and thyroid dysfunction and intellectual impairment. The hormonal and intellectual deficits created by these therapies causes significant impairment of the survivors.^[17]

Epidemiology

Medulloblastomas affect just under 2 people per million per year, and affect children 10 times more than adults.^[18] Medulloblastoma is the second most frequent brain tumor in children after pilocytic astrocytoma^[19] and the most common malignant brain tumor in children, comprising 14.5% of newly diagnosed cases.^[20] In adults, medulloblastoma is rare, comprising fewer than 2% of CNS malignancies.^[21]

The rate of new cases of childhood medulloblastoma is higher in males (62%) than females (38%), a feature which is not seen in adults.^[18]^[22] Medulloblastoma and other PNET`s are more prevalent in younger children than older children. Forty percent of medulloblastoma patients are diagnosed before the age of 5, 31% are between the ages of 5 and 9, 18.3% are between the ages of 10 and 14, and 12.7% are between the ages of 15 and 19.^[23]

Research

Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis.^[24] The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10% of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 Large T- antigen, other than p53.^[25]

References

^ Hinz, Chris; Hesser, Deneen. Focusing On Brain Tumors: Medulloblastoma. American Brain Tumor Association. ISBN 0-944093-67-1. ^{[page needed]}
^ ^a ^b Smoll, Nicolas R. (2012). "Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs)". Cancer 118 (5): 1313–22. doi:10.1002/cncr.26387. PMID 21837678.
^ Roger Packer M.D, Medulloblastoma Clinical Trials and Noteworthy Treatments for Brain Tumors 2002.
^ White, Lucile E.; Levy, Ross M.; Alam, Murad (2008). "Ch. 127. Neoplasias and Hyperplasias of Muscular and Neural Origin". In Wolff K, Goldsmith LA, Katz SI, Gilchrest B, Paller AS, Leffell DJ. Fitzpatrick's Dermatology in General Medicine (7e ed.). McGraw-Hill Medical.
^ Ropper AH, Samuels MA. "Ch. 31. Intracranial Neoplasms and Paraneoplastic Disorders". In Ropper AH, Samuels MA. Adams and Victor's Principles of Neurology (9e ed.).
^ Taylor, Michael D.; Northcott, P. A.; Korshunov, A; Remke, M; Cho, Y. J.; Clifford, S. C.; Eberhart, C. G.; Parsons, D. W.; Rutkowski, S; Gajjar, A; Ellison, D. W.; Lichter, P; Gilbertson, R. J.; Pomeroy, S. L.; Kool, M; Pfister, S. M. (Feb 2012). "Molecular subgroups of medulloblastoma: the current consensus". Acta Neuropathologica 123 (4): 465–72. doi:10.1007/s00401-011-0922-z. PMC 3306779. PMID 22134537.
^ Dubuc, Adrian M.; Morrissy, A. Sorana; Kloosterhof, Nanne K.; Northcott, Paul A.; Yu, Emily P. Y.; Shih, David; Peacock, John; Grajkowska, Wieslawa; Van Meter, Timothy; Eberhart, Charles G.; Pfister, Stefan; Marra, Marco A.; Weiss, William A.; Scherer, Stephen W.; Rutka, James T.; French, Pim J.; Taylor, Michael D. (Feb 2012). "Subgroup-specific alternative splicing in medulloblastoma". Acta Neuropathologica 123 (4): 485. doi:10.1007/s00401-012-0959-7. PMID 22358458.
^ Jones, D. T.; Jäger, N; Kool, M; Zichner, T; Hutter, B; Sultan, M; Cho, Y. J.; Pugh, T. J.; Hovestadt, V; Stütz, A. M.; Rausch, T; Warnatz, H. J.; Ryzhova, M; Bender, S; Sturm, D; Pleier, S; Cin, H; Pfaff, E; Sieber, L; Wittmann, A; Remke, M; Witt, H; Hutter, S; Tzaridis, T; Weischenfeldt, J; Raeder, B; Avci, M; Amstislavskiy, V; Zapatka, M et al. (Jul 2012). "Dissecting the genomic complexity underlying medulloblastoma". Nature 488 (7409): 100–5. Bibcode:2012Natur.488..100J. doi:10.1038/nature11284. PMC 3662966. PMID 22832583.
^ Burger, Peter C.; Yu, I-T; Tihan, Tarik; Friedman, Henry S.; Strother, Douglas R.; Kepner, James L.; Duffner, Patricia K.; Kun, Larry E.; Perlman, Elizabeth J. (1998). "Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System: A Highly Malignant Tumor of Infancy and Childhood Frequently Mistaken for Medulloblastoma". The American Journal of Surgical Pathology 22 (9): 1083–92. doi:10.1097/00000478-199809000-00007. PMID 9737241.
^ Merchant, Thomas E.; Hua, Chia-ho; Shukla, Hemant; Ying, Xiaofei; Nill, Simeon; Oelfke, Uwe (2008). "Proton versus photon radiotherapy for common pediatric brain tumors: Comparison of models of dose characteristics and their relationship to cognitive function". Pediatric Blood & Cancer 51 (1): 110–7. doi:10.1002/pbc.21530. PMID 18306274.
^ Blomstrand, M; Brodin, N. P.; Munck Af Rosenschöld, P; Vogelius, I. R.; Sánchez Merino, G; Kiil-Berthlesen, A; Blomgren, K; Lannering, B; Bentzen, S. M.; Björk-Eriksson, T (2012). "Estimated clinical benefit of protecting neurogenesis in the developing brain during radiation therapy for pediatric medulloblastoma". Neuro-Oncology 14 (7): 882–9. doi:10.1093/neuonc/nos120. PMC 3379806. PMID 22611031.
^ Michiels, Erna MC; Schouten-Van Meeteren, Antoinette YN; Doz, François; Janssens, Geert O; Van Dalen, Elvira C (2015). "Chemotherapy for children with medulloblastoma". Cochrane Database of Systematic Reviews 1: CD006678. doi:10.1002/14651858.CD006678.pub2. PMID 25553354.
^ Fossati, Piero; Ricardi, Umberto; Orecchia, Roberto (February 2009). "Pediatric medulloblastoma: Toxicity of current treatment and potential role of protontherapy". Cancer Treatment Reviews 35 (1): 79–96. doi:10.1016/j.ctrv.2008.09.002.
^ Crawford, John R; MacDonald, Tobey J; Packer, Roger J (December 2007). "Medulloblastoma in childhood: new biological advances". The Lancet Neurology 6 (12): 1073–1085. doi:10.1016/S1474-4422(07)70289-2.
^ Pfister, S.; Remke, M.; Benner, A.; Mendrzyk, F.; Toedt, G.; Felsberg, J.; Wittmann, A.; Devens, F.; Gerber, N. U.; Joos, S.; Kulozik, A.; Reifenberger, G.; Rutkowski, S.; Wiestler, O. D.; Radlwimmer, B.; Scheurlen, W.; Lichter, P.; Korshunov, A. (2009). "Outcome Prediction in Pediatric Medulloblastoma Based on DNA Copy-Number Aberrations of Chromosomes 6q and 17q and the MYC and MYCN Loci". Journal of Clinical Oncology 27 (10): 1627. doi:10.1200/JCO.2008.17.9432. PMID 19255330.
^ Taylor, Michael D.; Northcott, Paul A.; Korshunov, Andrey; Remke, Marc; Cho, Yoon-Jae; Clifford, Steven C.; Eberhart, Charles G.; Parsons, D. Williams; Rutkowski, Stefan; Gajjar, Amar; Ellison, David W.; Lichter, Peter; Gilbertson, Richard J.; Pomeroy, Scott L.; Kool, Marcel; Pfister, Stefan M. (2011). "Molecular subgroups of medulloblastoma: The current consensus". Acta Neuropathologica 123 (4): 465. doi:10.1007/s00401-011-0922-z. PMID 22134537.
^ Packer, Roger J. (2010). "Medulloblastoma". ^{[self-published source?]}
^ ^a ^b Smoll, Nicolas R.; Drummond, Katharine J. (2012). "The incidence of medulloblastomas and primitive neurectodermal tumours in adults and children". Journal of Clinical Neuroscience 19 (11): 1541. doi:10.1016/j.jocn.2012.04.009. PMID 22981874.
^ http://neuropathology-web.org/chapter7/chapter7cMedulloblastoma.html
^ Gurney, James G.; Smith, Malcolm A.; Bunin, Greta R. (1999). "CNS and Miscellaneous Intracranial and Intraspinal Neoplasms" (PDF). In Ries LAG, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, Bunin GR. Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975–1995 (PDF). Bethesda MD: National Cancer Institute. NIH Pub. No. 99-4649.
^ Selected Primary Brain and Central Nervous System Tumor Age-Specific Incidence Rates, Central Brain Tumor Registry of the United States, 1998–2002.
^ Selected Childhood Primary Brain and Central Nervous System Tumor Incidence Rates by Major Histology Groupings, Histology and Gender Central Brain Tumor Registry of the United States, 1998–2002.
^ Selected Childhood Primary Brain and Central Nervous System Tumor Age-Specific Incidence Rates, Central Brain Tumor Registry of the United States, 1998–2002.
^ Eibl, R. H.; Kleihues, P; Jat, P. S.; Wiestler, O. D. (March 1994). "A model for primitive neuroectodermal tumors in transgenic neural transplants harboring the SV40 large T antigen". The American journal of pathology 144 (3): 556–64. PMC 1887088. PMID 8129041.
^ Ohgaki, H; Eibl, R. H.; Wiestler, O. D.; Yasargil, M. G.; Newcomb, E. W.; Kleihues, P (November 1991). "P53 mutations in nonastrocytic human brain tumors". Cancer research 51 (22): 6202–5. PMID 1933879.

External links

Brain and Spinal Tumors: Hope Through Research (National Institute of Neurological Disorders and Stroke)
I'll get better tomorrow (2009) a documentary film following for 16 months the treatments of 3 children with medulloblastoma.
Medulloblastoma Images MedPix Medical Image Database
Cancer.Net: Medulloblastoma - Childhood

UpToDate Contents

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1. 髄芽腫の治療および予後 treatment and prognosis of medulloblastoma
2. 髄芽腫の臨床症状、診断、およびリスク層別化 clinical presentation diagnosis and risk stratification of medulloblastoma
3. 髄芽腫の組織病理および分子学的病因 histopathology and molecular pathogenesis of medulloblastoma
4. 小児における中枢神経系腫瘍の疫学 epidemiology of central nervous system tumors in children
5. 小児における中枢神経系腫瘍のマネージメントに関する概要 overview of the management of central nervous system tumors in children

English Journal

Downregulation of microRNA-431 by human interferon-β inhibits viability of medulloblastoma and glioblastoma cells via upregulation of SOCS6.

Tanaka T1, Arai M2, Jiang X2, Sugaya S1, Kanda T2, Fujii K3, Kita K1, Sugita K4, Imazeki F2, Miyashita T5, Kaneda A1, Yokosuka O2.Author information 1Department of Molecular Oncology, Graduate School of Medicine, Chiba University, Chiba 260-8670, Japan.2Department of Gastroenterology and Nephrology, Graduate School of Medicine, Chiba University, Chiba 260-8670, Japan.3Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba 260-8670, Japan.4Faculty of Education, Chiba University, Chiba 263-8522, Japan.5Department of Molecular Genetics, Kitasato University Graduate School of Medical Sciences, Minami-ku, Sagamihara, Kanagawa 252-0374, Japan.AbstractmiRNAs are small non-coding RNAs that inhibit gene expression by cleaving or hindering the translation of target mRNAs. In this study, we focused on miR-431, which mediated inhibition of cell viability by human interferon-β (HuIFN-β). We aimed to demonstrate an antineoplastic effect of HuIFN-β via miR-431 expression against medulloblastoma and glioblastoma, because HuIFN-β is frequently used in adjuvant therapy of these tumors. Addition of HuIFN-β to medulloblastoma and glioblastoma cells reduced viability, significantly decreased miR-431 expression, upregulated expression of SOCS6 (putative miR-431 target genes) and inhibited Janus kinase (JAK) 1 and signal transducer and activator of transcription (STAT) 2. The mitogen-activated protein kinase (MAPK) pathway, but not the phosphoinositide 3-kinase (PI3K)-Akt pathway, was downregulated in medulloblastoma cells, whereas the PI3K-Akt pathway, but not the MAPK pathway, was downregulated in glioblastoma cells. Addition of HuIFN-β and transient transfection with miR-431 to medulloblastoma and glioblastoma cells did not reduce viability, downregulated expression of SOCS6, and concomitantly activated the JAK1 and STAT2. We propose that, in medulloblastoma and glioblastoma cells, HuIFN-β decreases miR-431 expression and upregulates SOCS6 expression, and consequently inhibit cell proliferation by suppressing the JAK-STAT signaling pathway.
International journal of oncology.Int J Oncol.2014 May;44(5):1685-90. doi: 10.3892/ijo.2014.2317. Epub 2014 Feb 28.
miRNAs are small non-coding RNAs that inhibit gene expression by cleaving or hindering the translation of target mRNAs. In this study, we focused on miR-431, which mediated inhibition of cell viability by human interferon-β (HuIFN-β). We aimed to demonstrate an antineoplastic effect of HuIFN-β vi
PMID 24584142

High-dose busulfan-thiotepa with autologous stem cell transplantation followed by posterior fossa irradiation in young children with classical or incompletely resected medulloblastoma.

Bergthold G1, Kababri ME, Varlet P, Dhermain F, Sainte-Rose C, Raquin MA, Kieffer V, Goma G, Grill J, Valteau-Couanet D, Dufour C.Author information 1Department of Paediatric and Adolescent Oncology, Institut Gustave Roussy, Villejuif, France; American Memorial Hospital, CHU de Reims, Reims, France.AbstractBACKGROUND: The aim of the study is to evaluate the outcome of young children with high risk localized medulloblastomas (newly diagnosed classical or incompletely resected) treated by high-dose busulfan-thiotepa with autologous stem cell rescue (ASCT) followed by focal radiation therapy (RT).
Pediatric blood & cancer.Pediatr Blood Cancer.2014 May;61(5):907-12. doi: 10.1002/pbc.24954. Epub 2014 Jan 28.
BACKGROUND: The aim of the study is to evaluate the outcome of young children with high risk localized medulloblastomas (newly diagnosed classical or incompletely resected) treated by high-dose busulfan-thiotepa with autologous stem cell rescue (ASCT) followed by focal radiation therapy (RT).PROCEDU
PMID 24470384

Transitioning from genotypes to epigenotypes: Why the time has come for medulloblastoma epigenomics.

Batora NV1, Sturm D1, Jones DT1, Kool M1, Pfister SM2, Northcott PA3.Author information 1Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, Heidelberg 69120, Germany.2Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, Heidelberg 69120, Germany; Department of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Im Neuenheimer Feld 430, Heidelberg 69120, Germany.3Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, Heidelberg 69120, Germany. Electronic address: p.northcott@dkfz-heidelberg.de.AbstractRecent advances in genomic technologies have allowed for tremendous progress in our understanding of the biology underlying medulloblastoma, a malignant childhood brain tumor. Consensus molecular subgroups have been put forth by the pediatric neuro-oncology community and next-generation genomic studies have led to an improved description of driver genes and pathways somatically altered in these subgroups. In contrast to the impressive pace at which advances have been made at the level of the medulloblastoma genome, comparable studies of the epigenome have lagged behind. Complementary data yielded from genomic sequencing and copy number profiling have verified frequent targeting of chromatin modifiers in medulloblastoma, highly suggestive of prominent epigenetic deregulation in the disease. Past studies of DNA methylation-dependent gene silencing and microRNA expression analyses further support the concept of medulloblastoma as an epigenetic disease. In this Review, we aim to summarize the key findings of past reports pertaining to medulloblastoma epigenetics as well as recent and ongoing genomic efforts linking somatic alterations of the genome with inferred deregulation of the epigenome. In addition, we predict what is on the horizon for medulloblastoma epigenetics and how aberrant changes in the medulloblastoma epigenome might serve as an attractive target for future therapies.
Neuroscience.Neuroscience.2014 Apr 4;264C:171-185. doi: 10.1016/j.neuroscience.2013.07.030. Epub 2013 Jul 20.
Recent advances in genomic technologies have allowed for tremendous progress in our understanding of the biology underlying medulloblastoma, a malignant childhood brain tumor. Consensus molecular subgroups have been put forth by the pediatric neuro-oncology community and next-generation genomic stud
PMID 23876321

Japanese Journal

小脳性無言症の障害機序と予後に関する一考察 : 小脳髄芽腫術後に無言症を呈した児の言語訓練経過から

藤田隼平,川崎聡大,古西隆之
とやま発達福祉学年報 4, 29-33, 2013-05-31
小脳髄芽腫術後に小脳性無言症を呈した7歳の男児に対して言語訓練を実施した。本症例は発話表出のみならず全般的なコミュニケーション機能の低下と発動性の低下を呈したことと、水頭症や白質周囲の浮腫的病変から、視床や補足運動野の関与が考えられた。小脳性無言症から回復を見せた8か月後においても後遺症と思われる知的発達遅滞を認めたことからも、小脳性無言症を呈した児に関しては主徴候である表出面だけでなく、中長期的 …
NAID 120005290255

In vitro stemness characterization of radio-resistant clones isolated from a medulloblastoma cell line ONS-76

Sun Lue,Moritake Takashi,Zheng Yun-Wen,Suzuki Kenshi,Gerelchuluun Ariungerel,Hong Zhengshan,Zenkoh Junko,Taniguchi Hideki,Tsuboi Koji,盛武敬,坪井康次
Journal of radiation research 54(1), 61-69, 2013-01-00
… One-third of patients with medulloblastoma die due to recurrence after various treatments including radiotherapy. … Although it has been postulated that cancer stem-like cells are radio-resistant and play an important role in tumor recurrence, the "stemness" of medulloblastoma cells surviving irradiation has not yet been elucidated. … Using a medulloblastoma cell line ONS-76, cells that survived gamma irradiation were investigated on their "stemness" in vitro. …
NAID 120005246509

44. Medulloblastoma加療後に発症した巨大卵巣腫瘍の1例(一般演題,第47回日本小児外科学会関東甲信越地方会)

神保教広,瓜田泰久,高安肇,新開統子,藤代準,五藤周,上杉達,坂元直哉,佐々木理人,増本幸二
日本小児外科学会雑誌 48(7), 1083-1084, 2012-12-20
NAID 110009562310

Related Pictures

★リンクテーブル★

国試過去問	「093A079」
リンク元	「脳腫瘍」「髄芽腫」「medullomyoblastoma」
拡張検索	「desmoplastic medulloblastoma」「childhood medulloblastoma」

「093A079」

Medulloblastoma
	CT scan, showing a tumorous mass in the posterior fossa, giving rise to obstructive hydrocephalus, in a six-year-old girl
Classification and external resources
Specialty	Oncology
ICD-O	M9470/3
OMIM	155255
DiseasesDB	31105
eMedicine	neuro/624 ped/1396 radio/434
MeSH	D008527

　　[★]

放射線感受性の高い脳腫瘍はどれ？

a. (1)(2)
b. (1)(5)
c. (2)(3)
d. (3)(4)
e. (4)(5)

「脳腫瘍」

　　[★]

英: brain tumor, cerebral tumor
同: 脳新生物 brain neoplasm
関: 頭蓋内腫瘍

概念

分類

原発性脳腫瘍

神経膠腫 glioma

星状細胞腫 astrocytoma

胚腫 germinoma

転移性脳腫瘍

疫学

発生率：10万人対10-15人。罹患率：10万人対46人　→

腫瘍別発生頻度

腫瘍別発生頻度		小児	成人
神経膠腫	33%	星状細胞腫	髄膜腫
髄膜腫	22%	髄芽腫	膠芽腫
下垂体腺腫	15%	頭蓋咽頭腫	下垂体腺腫
神経鞘腫	9%	胚細胞腫	神経鞘腫
頭蓋咽頭腫	5%	上衣腫	転移性脳腫瘍

YN.J.188

部位	種類	小児	成人
頭蓋骨	頭蓋骨腫瘍	○	○
大脳半球	神経膠腫		○
大脳半球	髄膜腫		○
松果体	胚細胞腫	○
小脳半球	星細胞腫	○
小脳半球	血管芽腫		○
小脳虫部	髄芽腫	○
第四脳室	上衣腫	○
鞍上部・視交叉部・下垂体部	頭蓋咽頭腫	○
	視神経膠腫	○
	胚細胞腫	○
	下垂体腺腫		○
	髄膜腫		○
小脳橋角部	聴神経鞘腫		○
脳幹部	神経膠腫	○	○

小児の脳腫瘍

SCN.173

腫瘍：星細胞腫、髄芽腫、頭蓋咽頭腫、胚細胞腫、上衣腫の順に多い。
部位：1歳まではテント上、2-7歳まではテント下、8-15歳まではテント上に多い。

SRA.163

	後発年齢	好発部位
小脳星細胞腫	5～10歳	小脳半球
髄芽腫	5～10歳(男児に多い)	小脳虫部から発生
頭蓋咽頭腫	10～15歳	トルコ鞍上部
上衣腫	10～15,30～40歳	第四脳室、側脳室
髄芽腫	10～30歳	松果体部、トルコ鞍上部
脳幹部膠腫	～15歳	橋
視神経膠腫	～15歳	視神経視交叉